Subacute sclerosing panencephalitis

Garg, Ravindra Kumar

doi:10.1136/pmj.78.916.63

Cited by 316 publications

(179 citation statements)

References 87 publications

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“…sclerosing panencephalitis (SSPE) (25). Analysis of viral RNA from SSPE autopsies reveals extensive A-to-I hypermutation in the matrix gene and several other virus genes (10,26,27), implicating a role for ADAR during MV infection.…”

mentioning

confidence: 99%

RNA-specific Adenosine Deaminase ADAR1 Suppresses Measles Virus-induced Apoptosis and Activation of Protein Kinase PKR

Toth

Cattaneo

et al. 2009

Journal of Biological Chemistry

137

193

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ADAR1 (adenosine deaminase acting on RNA) catalyzes the conversion of adenosine to inosine, a process known as A-to-I editing. Extensive A-to-I editing has been described in viral RNAs isolated from the brains of patients persistently infected with measles virus, although the precise role of ADAR during measles virus infection remains unknown. We generated human HeLa cells stably deficient in ADAR1 ("ADAR1 kd cells") through short hairpin RNA-mediated knockdown, and using these cells, we tested the effect of ADAR1 deficiency on measles virus (MVvac strain) growth and virus-induced cell death. We found that the growth of mutant viruses lacking expression of the viral accessory proteins V and C (V ko and C ko , respectively) was decreased in ADAR1-deficient cells compared with ADAR1-sufficient cells. In addition, apoptosis was enhanced in ADAR1-deficient cells following infection with wild type and V ko virus but not following infection with C ko virus or treatment with tumor necrosis factor-␣ or staurosporine. Furthermore, in C ko -infected ADAR1-sufficient cells when ADAR1 did not protect against apoptosis, caspase cleavage of the ADAR1 p150 protein was detected. Finally, enhanced apoptosis in ADAR1 kd cells following infection with wild type and V ko virus correlated with enhanced activation of PKR kinase and interferon regulatory factor IRF-3. Taken together, these results demonstrate that ADAR1 is a proviral, antiapoptotic host factor in the context of measles virus infection and suggest that the antiapoptotic activity of ADAR1 is achieved through suppression of activation of proapoptotic and double-stranded RNA-dependent activities, as exemplified by PKR and IRF-3.

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mentioning

confidence: 99%

RNA-specific Adenosine Deaminase ADAR1 Suppresses Measles Virus-induced Apoptosis and Activation of Protein Kinase PKR

Toth

Cattaneo

et al. 2009

Journal of Biological Chemistry

137

193

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“…Measles infection in children younger than 1 year of age poses a risk for SSPE 16 times greater than in those aged 5 years or older (8). SSPE can occur an average 7 years post-measles infection (7).…”

Section: Discussionmentioning

confidence: 99%

Hemidistoni ile Prezente Olan Subakut Sklerozan Panensefalit

Serin¹,

Bilen²,

Cansu³

2014

Haseki

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“…A very few patients had shown the typical periodic slow wave complex (PSWC) in EEG before intervention. Benzodiazepine certainly played a good role in eliciting the typical periodic slow wave complex (PSWC) in these patients [2]. Both the benzodiazepines made these characteristic changes even in the initial normal EEG cases.…”

Section: Discussionmentioning

confidence: 99%

“…This is characterized by cognitive and behavioral deterioration with atonic and/ or myoclonic seizures; furthermore high titer of measles antibody in cerebrospinal fluid and typical periodic complexes on electroencephalogram (EEG) are the most suggestive investigational findings of SSPE [2]. The typical periodic complexes which are recorded in SSPE are composed of bilaterally symmetrical, synchronous, high voltage bursts of polyphasic, stereotyped delta waves.…”

Section: Introductionmentioning

confidence: 99%

Role of Routine Benzodiazepin in Eliciting Classical Electroencephalogram (EEG) Response in Suspected Sub-Acute Sclerosing Panencephalitis (SSPE) Cases

Chowdhury¹,

Saha²,

Yusuf³

et al. 2017

WJNS

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Background: EEG could be normal or atypical in spite of suggestive clinical features and positive measles Ab of SSPE cases which could have typical EEG pattern after Benzodiazepine. Objectives: The purpose of the present study was to find out the necessity of administration of benzodiazepine during EEG recording of SSPE cases as well as to compare the efficacy of diazepam and midazolam in eliciting EEG pattern. Methodology: This double blind, parallel, single centered, non-randomized clinical trial was conducted in the Department of Pediatric Neurology at National Institute of Neurosciences, Dhaka, Bangladesh from July 2014 to June 2015 for a period of 1 (one) year. All the clinical and investigational suspected cases of sub-acute Sclerosing Panencephalitis (SSPE) children in both sexes were included as study population. Others neurodegenerative diseases including Wilson's disease were excluded from this study. Patients were divided into two groups named as group A who were given diazepam and the other group B was given midazolam in IV during EEG recording. The clinical outcomes were measured and were recorded in a pre-designed data sheet. Result: The characteristic typical periodic slow wave complex (PSWC) was found only in 8 (30.8%) patients among the 26 (100.0%) before intervention with benzodiazepines. The remaining 18 (69.2%) had non-typical PSWC of which 10 (38.5%) were normal, 3 (11.5%) with atypical PSWC and 5 (19.2%) were with other EEG findings. After intervention with benzodiazepines, 23 (88.5%) had shown typical PSWC and only 3 (11.5%) had non-typical PSWC. Among the typical PSWC cases after interHow to cite this paper: Chowdhury, Y

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Subacute sclerosing panencephalitis

Cited by 316 publications

References 87 publications

RNA-specific Adenosine Deaminase ADAR1 Suppresses Measles Virus-induced Apoptosis and Activation of Protein Kinase PKR

RNA-specific Adenosine Deaminase ADAR1 Suppresses Measles Virus-induced Apoptosis and Activation of Protein Kinase PKR

Hemidistoni ile Prezente Olan Subakut Sklerozan Panensefalit

Role of Routine Benzodiazepin in Eliciting Classical Electroencephalogram (EEG) Response in Suspected Sub-Acute Sclerosing Panencephalitis (SSPE) Cases

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